Uveal melanoma: epidemiology, etiology, and treatment of primary disease

Benjamin A Krantz,1 Nikita Dave,2 Kimberly M Komatsubara,2 Brian P Marr,3,4 Richard D Carvajal5 1Division of Hospital Medicine, 2Division of Hematology/Oncology, Columbia University Medical Center, 3Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, 4Department of Ophthalmology, W...

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Autores principales:	Krantz BA, Dave N, Komatsubara KM, Marr BP, Carvajal RD
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2017
Materias:	Uveal Melanoma Ocular Melanoma GNAQ GNA11 MAP Kinase MEK Ophthalmology RE1-994
Acceso en línea:	https://doaj.org/article/d477b246766a4bcdab97755a87251238
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spelling	oai:doaj.org-article:d477b246766a4bcdab97755a872512382021-12-02T00:34:37ZUveal melanoma: epidemiology, etiology, and treatment of primary disease1177-5483https://doaj.org/article/d477b246766a4bcdab97755a872512382017-01-01T00:00:00Zhttps://www.dovepress.com/uveal-melanoma-epidemiology-etiology-and-treatment-of-primary-disease-peer-reviewed-article-OPTHhttps://doaj.org/toc/1177-5483Benjamin A Krantz,1 Nikita Dave,2 Kimberly M Komatsubara,2 Brian P Marr,3,4 Richard D Carvajal5 1Division of Hospital Medicine, 2Division of Hematology/Oncology, Columbia University Medical Center, 3Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, 4Department of Ophthalmology, Weill Cornell Medical College, 5Division of Hematology/Oncology, Columbia University Medical Center, New York, NY, USA Abstract: Uveal melanoma (UM) is the most common intraocular malignancy and arises from melanocytes in the iris, ciliary body, or choroid. Early diagnosis and local treatment is crucial, as survival correlates with primary tumor size. However, approximately 50% of patients will develop metastatic disease with 6–12 months’ survival from metastatic diagnosis. Genomic analyses have led to the development of gene-expression profiles that effectively predict metastatic progression; unfortunately, no adjuvant therapy has been shown to prolong survival to date. New insights into the molecular biology of UM have found frequent activating mutations in genes encoding for the G-protein α-subunit, GNAQ and GNA11, and improved understanding of the downstream signaling pathways MAPK, PI3K/Akt, and Hippo have afforded an array of new targets for treatment of this disease. Studies are under way with rationally developed regimens targeting these pathways, and novel agents are under development. We review the diagnosis, management, and surveillance of primary UM and the adjuvant therapy trials under way. Keywords: uveal melanoma, ocular melanoma, GNAQ, GNA11, MAP kinase, MEKKrantz BADave NKomatsubara KMMarr BPCarvajal RDDove Medical PressarticleUveal MelanomaOcular MelanomaGNAQGNA11MAP KinaseMEKOphthalmologyRE1-994ENClinical Ophthalmology, Vol Volume 11, Pp 279-289 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Uveal Melanoma Ocular Melanoma GNAQ GNA11 MAP Kinase MEK Ophthalmology RE1-994
spellingShingle	Uveal Melanoma Ocular Melanoma GNAQ GNA11 MAP Kinase MEK Ophthalmology RE1-994 Krantz BA Dave N Komatsubara KM Marr BP Carvajal RD Uveal melanoma: epidemiology, etiology, and treatment of primary disease
description	Benjamin A Krantz,1 Nikita Dave,2 Kimberly M Komatsubara,2 Brian P Marr,3,4 Richard D Carvajal5 1Division of Hospital Medicine, 2Division of Hematology/Oncology, Columbia University Medical Center, 3Ophthalmic Oncology Service, Memorial Sloan Kettering Cancer Center, 4Department of Ophthalmology, Weill Cornell Medical College, 5Division of Hematology/Oncology, Columbia University Medical Center, New York, NY, USA Abstract: Uveal melanoma (UM) is the most common intraocular malignancy and arises from melanocytes in the iris, ciliary body, or choroid. Early diagnosis and local treatment is crucial, as survival correlates with primary tumor size. However, approximately 50% of patients will develop metastatic disease with 6–12 months’ survival from metastatic diagnosis. Genomic analyses have led to the development of gene-expression profiles that effectively predict metastatic progression; unfortunately, no adjuvant therapy has been shown to prolong survival to date. New insights into the molecular biology of UM have found frequent activating mutations in genes encoding for the G-protein α-subunit, GNAQ and GNA11, and improved understanding of the downstream signaling pathways MAPK, PI3K/Akt, and Hippo have afforded an array of new targets for treatment of this disease. Studies are under way with rationally developed regimens targeting these pathways, and novel agents are under development. We review the diagnosis, management, and surveillance of primary UM and the adjuvant therapy trials under way. Keywords: uveal melanoma, ocular melanoma, GNAQ, GNA11, MAP kinase, MEK
format	article
author	Krantz BA Dave N Komatsubara KM Marr BP Carvajal RD
author_facet	Krantz BA Dave N Komatsubara KM Marr BP Carvajal RD
author_sort	Krantz BA
title	Uveal melanoma: epidemiology, etiology, and treatment of primary disease
title_short	Uveal melanoma: epidemiology, etiology, and treatment of primary disease
title_full	Uveal melanoma: epidemiology, etiology, and treatment of primary disease
title_fullStr	Uveal melanoma: epidemiology, etiology, and treatment of primary disease
title_full_unstemmed	Uveal melanoma: epidemiology, etiology, and treatment of primary disease
title_sort	uveal melanoma: epidemiology, etiology, and treatment of primary disease
publisher	Dove Medical Press
publishDate	2017
url	https://doaj.org/article/d477b246766a4bcdab97755a87251238
work_keys_str_mv	AT krantzba uvealmelanomaepidemiologyetiologyandtreatmentofprimarydisease AT daven uvealmelanomaepidemiologyetiologyandtreatmentofprimarydisease AT komatsubarakm uvealmelanomaepidemiologyetiologyandtreatmentofprimarydisease AT marrbp uvealmelanomaepidemiologyetiologyandtreatmentofprimarydisease AT carvajalrd uvealmelanomaepidemiologyetiologyandtreatmentofprimarydisease
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Uveal melanoma: epidemiology, etiology, and treatment of primary disease

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